Dermatology Online Journal

Acquired lymphangiectasia of the vulva is very uncommon. Owing to the non-specific papillomatous manifestation and the vast array of possible differential diagnoses, lymphangioma circumscriptum (LC) still presents a diagnostic challenge. In this report, we present a very rare form of acquired vulvar LC in a 71-year-old patient with a longstanding history of asymptomatic lesions over the labia majora that had been previously treated as genital warts. On examination, the patient had multiple clustered translucent papules up to 15mm in diameter, morphologically reminiscent of vesicles, that oozed clear fluid throughout her groin and swollen labia majora. The patient also suffered concomitant bilateral lower-extremity lymphedema. A skin biopsy showed multiple, irregular-shaped lumina containing eosinophilic material in the upper dermis. Dilated lymphatic channels were lined by a single layer of flattened endothelial cells and the overlying epidermis showed acanthosis, hyperkeratosis, focal mild pseudoepitheliomatous hyperplasia. There is still no consensus on the optimal management of LC. Our patient was referred to a plastic surgeon for further evaluation and treatment. Although there are a variety of therapeutic modalities for LC, positive results are few and relapses are observed.